Chii chinonzi Gorlin Syndrome? Usazvidya moyo, ngatitaurei nezvazvo zvakadzama!

Wakamboona mavara matsva kana mapundu achigara achiumba paganda rako here? Kana kuti wakambonzwa nezvemapundu asingarwadzi pashaya yako here? Izvi zvinogona kunge zvakajairika. Zvisinei, mune zvisingawanzoitiki, zviratidzo izvi zvinogona kunge zvine chekuita nechirwere chemajini chisingawanzoitiki. Ndiyo mamiriro atichataura nezvawo nhasi, anonzi Gorlin Syndrome. Usatye paunonzwa zita iri. Ngatitaure nezvazvo zviri nyore, nenzira yaunogona kunzwisisa.

Chii chaizvo chinonzi Gorlin Syndrome?

Zvichitaurwa zviri nyore, Gorlin syndrome chirwere chisingawanzoitiki chemajini . Chinozivikanwawo nemamwe mazita, akadai sekuti `(Basal Cell Nevus Syndrome)`, `(Nevoid Basal Cell Carcinoma Syndrome - NBCCS)` uye `(Gorlin-Goltz Syndrome)`. Munhu ane chirwere ichi ane mukana wakawedzerwa wekuva nemabundu ekenza uye asiri ekenza (zvichireva kuti, asingakuvadzi muviri zvakanyanya). Kunyanya, kune mukana wakawedzerwa wekuva nerudzi rwekenza yeganda inonzi `(Basal Cell Carcinoma)` . Iyi ndiyo mhando yekenza yeganda yakajairika.

Fungidzira, kune mamwe majini mumuviri wedu, ayo basa guru nderekudzora kupatsanurwa kwemasero kusingakoshi uye kuumbwa kwemabundu. Tinodaidzawo aya kuti "majini anodzvinyirira bundu". Saka, muGorlin syndrome, chinoitika ndechekuti pane shanduko, kureva kuti, "mutation", mune rimwe remajini aya. Ndokusaka njodzi yekukura nemabundu ichikwira sezvataurwa.

Chinhu chinonyanya kukosha ndechekuti hapana mushonga chaiwo weGorlin syndrome parizvino . Asi musazvidya moyo! Kana mukatevera mirairo yachiremba wenyu nemazvo, uye kana zvinhu zvakaita sekenza zvikaonekwa uye zvikarapwa nekukurumidza , munhu ane chirwere ichi anogona kurarama hupenyu hwakajairika. Hazvifanirwe kukanganisa hupenyu hwenyu kana mhando yehupenyu hwenyu.

Dambudziko iri rakajairika zvakadii?

Gorlin syndrome chirwere chisingawanzoitiki . Nyanzvi dzinoti kunyangwe munyika yakaita seUnited States, chirwere ichi chinobata vanhu vasingasviki 50,000. Zvisinei, nhamba iyi inogona kunge yakakwira zvakanyanya. Chikonzero ndechekuti vamwe vanhu vane zviratidzo zvishoma zvekuti havatombozivi kuti vane chirwere ichi. Kazhinji, zviratidzo zvinoonekwa muvechidiki kana vechidiki .

Ndezvipi zviratidzo zveGorlin Syndrome?

Zviratidzo zvechirwere ichi zvinogona kusiyana kubva kumunhu kuenda kumunhu, asi pane zvimwe zviratidzo zvakajairika:

Zvinhu zvinonyanya kuonekwa

• Kansa dzeganda dzakawanda: Rudzi urwu rwegomarara reganda runowanzoonekwa munzvimbo dzine zuva dzakadai sekumeso nemutsipa. Zvisinei, kune vanhu vane chirwere cheGorlin, izvi zvinogona kuitika vachiri vadiki, vari mumakore avo ekuyaruka kana kutanga kwemakore ekuma20 .
• Makeratocysts eganda:Aya mabundu asingarwadzi, asingakonzere kenza anoumbwa mushaya. Anonyanya kuwanikwa muvana nevechidiki vane chirwere cheGorlin.
• Makomba madiki kana kuti maburi ari pazvanza nepasi petsoka: Izvi zvinonyanya kuonekwa mumakore ekuma1920. Izvi zvinogara zviripo.
• Kuchinja kwemafupa: Kumwe kuchinja kunogona kuonekwa mumaumbirwo emambabvu nemusana.

Zviratidzo zvisingawanzoitiki

Havasi vese vanozosangana nezviratidzo izvi, asi vamwe vanhu vanogona kusangana neizvi:

• Matumbu emuuropi: Aya anogona kuva kenza (medulloblastoma) kana kuti asiri kenza (meningioma).
• Mafibroma emwoyo kana maovari: Aya mabundu asina kenza, kazhinji asingakuvadzi.
• Matambudziko emaziso: zvinhu zvakaita se strabismus, nystagmus, uye cataracts.
• Kuchinja kwakanyanya mumapfupa: Semuenzaniso, kushaya zvachose musana (spina bifida).
• Zvimiro zvechiso zvisina kujairika: Kureba pakati pemaziso (hypertelorism), kuzvarwa uine muromo wakatsemuka/mukanwa wakatsemuka, uye madodzi machena madiki (milia) pachiso.
• Musoro wakakura kupfuura wakajairika (macrocephaly).
• Kuremara kwepfungwa.
• Mamiriro epfari `(Kubatwa nepfari)`.

Chii chinokonzera chirwere cheGorlin?

Sezvatakambokurukura, chirwere ichi chinokonzerwa nekuchinja kwemajini edu. Kutaura chokwadi, kune majini matatu mumuviri wedu anomisa kuumbwa kwemabundu (majini anodzvinyirira bundu). Ndiwo majini anonzi ``PTCH1`` (iri ndiro rinonyanya kubatwa), ``PTCH2`` uye ``SUFU``. Kana majini aya asingashande zvakanaka, mamwe masero anotanga kukura zvisina kujairika. Ndizvo zvinokonzera zviratidzo zvakaita semabundu.

Vanhu vazhinji vanogara nhaka iyi gene mutation kubva kuvabereki vavo. Zvisinei, mune dzimwe nguva, iyi gene mutation inogona kuitika zvisina tsarukano vasati vaberekwa, pasina nhoroondo yemhuri.

Kuchinja kwemajini uku kunogarwa nhaka nenzira inonzi "autosomal dominant" . Zvichitaurwa zviri nyore, unowana jini rakajairika kubva kune mumwe mubereki uye jini rakachinja kubva kune mumwe. Unofanira kuva nekuchinja kwemajini kwePTCH kana SUFU kuti uwane Gorlin syndrome.

Masayendisiti anoti munhu ane chirwere cheGorlin syndrome anongowana kenza chete kana kopi yenguva dzose yejini rinodzvinyirira bundu ikachinjwa neimwe nzira. Semuenzaniso, kukuvara kunobva muzuva kunogona kukonzera kuti jini rakanaka rishanduke. Zvadaro, majini ese ari maviri haashande zvakanaka, uye bundu harigone kumiswa. Izvi ndizvo zvinokonzera kenza yeganda. Uye kana mamwe masero mumuviri akakuvara nenzira iyi, mamwe marudzi ekenza anogona kukura.

Vanachiremba vanoongorora sei chirwere ichi?

Kuti aongorore chirwere cheGorlin, chiremba wako achaita zvinotevera:

• Kuongororwa kwemuviri: Tarisa kenza yeganda.
• Kuongororwa kwemifananidzo: Tarisa kana paine bundu, fibromas, kana matambudziko emapfupa mupfupa reshaya (semuenzaniso, X-ray, CT scan, MRI scan).
• Kuongororwa kwemuviri: Kuongororwa kweganda nemuviri: Nzira yekubviswa kwenyama diki kubva paganda kana pabundu roongororwa nemaikorosikopu kuti rione kana riine masero ekenza.
• Kuongororwa kwemajini: Ropa rinotorwa kuti rionekwe kana mutsauko wemajini wakabatana neGorlin syndrome.

Ndeipi mishonga yekurapa Gorlin Syndrome?

Kunyangwe pasina mushonga chaiwo weGorlin syndrome pachayo, kana ukaita bundu rine kenza kana kuti rinokonzera mamwe matambudziko, ungangoda kurirapa. Semuenzaniso:

• Gomarara reganda kana bundu repfupa remushaya rinogona kubviswa nekuvhiyiwa .
• Mimwe mishonga yekurapa kenza, yakaita sekirimu yemudumbu kana kurapa nechiedza chezuva, inogona kushandiswawo kuparadza masero ekenza.

Nekuti une njodzi yakakura yekubatwa nekenza, chiremba wako anogona kukukurudzira kuti ushandise mafuta akanaka ekudzivirira zuva, upfeke mbatya dzine maoko marefu, uye upfeke ngowani paunenge uri muzuva. Zvakare, bvunzo dzakadai seX-ray dzinoitwa chete kana zvichidikanwa. Kazhinji, vanachiremba vekenza vanodzivisa kupa radiation therapy sekurapa kenza kune vanhu vane Gorlin syndrome. Izvi zvinodaro nekuti radiation inogona kuwedzera njodzi yekenza.

Gorlin Syndrome inogona kudzivirirwa here?

Hatigone kudzora majini atinozvarwa nawo. Saka, hapana nzira yekudzivirira Gorlin syndrome. Zvisinei, unogona kuderedza njodzi yako yekubatwa nekenza yeganda nekutora matanho ekudzivirira . Zvakare, nekugara uchiongororwa nachiremba, unogona kuona kenza ichiri kutanga. Ipapo zvinova nyore kurapa.

Kana uine chirwere cheGorlin (kana kuti mumwe munhu mumhuri mako anacho) uye uri kuronga kuva nevana uye uchinetseka kuti mwana wako anogona kuchigara nhaka, ona chiremba wemajini . Vachakutsanangurira njodzi dzacho iwe nemumwe wako.

Ramangwana richava rakaita sei kune munhu anorarama nechirwere ichi? (Outlook)

Vanhu vazhinji vanokwanisa kugadzirisa chirwere cheGorlin nekutora matanho ekudzivirira kenza yeganda uye kuongororwa nachiremba nguva nenguva. Nekuongororwa kenza nguva dzose sezvakakurudzirwa nachiremba wako, chero matambudziko anogona kuonekwa nekukurumidza . Vanhu vane chirwere cheGorlin vanogona kurarama kwenguva yakareba uye pamwe chete nevasina chirwere ichi nevanhu vasina chirwere ichi.

Uyewo, chinhu chakakosha kurangarira ndechekuti havasi vese vane Gorlin syndrome vanozobatwa nekenza kana kuti vane matambudziko akafanana. Zvinosiyana kubva kumunhu kusvika kumunhu. Chiremba wako achakupa mazano ehutano hwako zvichienderana nezviratidzo zvako.

Ndinozvitarisira sei? (Kuzvitarisira)

Kana uine Gorlin syndrome, unofanira kutora matanho aya ekudzivirira kenza yeganda:

• Dzivisa kushandisa mibhedha yekuchenesa ganda.
• Gara mumba zvakanyanya sezvinobvira panguva yekubuda kwezuva (10am kusvika 4pm).
• Pfeka mafuta ekudzivirira zuva ane huwandu hwakakura hweSPF 30 zuva rega rega. Dzorerazve mafuta aya kakawanda.
• Paunenge uchibuda panze, pfeka mbatya dzinodzivirira UV uye ngowani ine muromo wakafara.

Uchafanirawo kuona vanachiremba vakasiyana-siyana kuti vaongorore kenza nezvimwe zvirwere. Kuti unofanira kuenda kangani zvinoenderana nekuti vanowana bundu here kana zvimwe zvirwere. Kushanya uku kwachiremba kunogona kusanganisira:

• Enda kunoongororwa ganda rako nachiremba weganda.
• Kuongororwa kwemazino uye kuongororwa kwemifananidzo yemazino kuti vaone kana paine bundu mupfupa reshaya.
• Kuongororwa kwemifananidzo yekuongorora fibromas, bundu remuuropi, kana matambudziko ekuona.

Ndinofanira kuona chiremba rini?

Kana muchifunga kuti imi kana mwana wenyu mune basal cell carcinoma, kurumidzai kuonana nachiremba . Ichi ndicho chiratidzo chinowanzoonekwa cheGorlin syndrome. Sezvakaita mamwe marudzi ekenza yeganda, zivai nezvemapundu matsva, anogara aripo, kana kuti anokura.

Gomarara reganda hariwanzobatanidzwa neGorlin syndrome, asi zvisinei nekuti chii chinokonzera, zvakakosha kuti munhu aongororwe.

Mibvunzo ipi yaunofanira kubvunza chiremba?

Kana ukaonekwa uine chirwere cheGorlin, ungada kubvunza chiremba wako mibvunzo iyi:

• Ndezvipi zviratidzo zvegomarara zvandinofanira kuziva?
• Kangani pandinofanira kuongororwa kenza?
• Ndingaitei kuti ndideredze njodzi yangu yekenza?
• Ndichada kurapwa kwegomarara risiri rekenza here?
• Mikana yekuti ndive nemwana ane chirwere cheGorlin ndeipi?

Gorlin syndrome inouya nematambudziko akati wandei ayo vanhu vasina chirwere ichi vangasasangana nawo. Kana uine NBCCS, iwe nechikwata chako chekurapa munofanira kungwarira zvikuru pakuona nekurapa chero basal cell carcinoma inokura.

Mharidzo Yekupedzisira Yekuenda Kumba

Mashoko akanaka ndeekuti basal cell carcinomas inorapika zvachose kana ikarapwa nekukasika. Zvakare, bundu risiri rekenza hariwanzo kukonzera matambudziko. Kana rikadaro, chiremba anogona kuribvisa kana kukurudzira mamwe marapirwo. Unogona kurarama hupenyu hurefu, hwakazara neGorlin syndrome. Saka, usavhunduke, asi chenjera. Tevedzera mazano akakodzera ekurapa, uye zvese zvichave zvakanaka!

Gorlin syndrome, NBCCS, basal cell carcinoma, zvirwere zvemajini, kenza yeganda, dermatology